Children with a Wolff–Parkinson–White electrocardiography pattern are usually asymptomatic. In these patients, the initial evaluations of organic cardiac disease and differentiation from fasciculoventricular pathways are important. If atrioventricular accessory pathways exist, the patient can develop atrioventricular reentrant tachycardia, rarely preexcited atrial fibrillation with rapid ventricular responses, sudden cardiac death, and heart failure caused by ventricular dyssynchrony. Risk stratification of fatal arrhythmic events by using noninvasive tests is thought to be difficult. The incidence of complications after catheter ablation is higher in smaller children. The acute effect, recurrence, and complication rates of catheter ablation are related to the location of the accessory pathway. Management is determined by the balance between the potential cardiovascular risks of accessory pathways and the possible complications of electrophysiological study and catheter ablation.

Despite deeper understanding of the pathophysiology of pulmonary artery hypertension (PAH), idiopathic and heritable PAH remains fatal. Specific therapies have advanced the clinical management of this challenging disease. Clinical trials have provided evidence of the effectiveness of targeted therapies, but data are limited for pediatric patients with PAH. Potential therapeutic approaches have been assessed, such as new pulmonary vasodilators, cell-based therapy and devices. The present investigation describes the novel and potential treatments that are available or under development. This paper provides an overview of the novel treatment options for PAH.

Restrictive cardiomyopathy (RCM) is characterized by impaired ventricular diastolic function with preserved ventricular contraction and wall thickness. The prognosis of young children with idiopathic RCM is poor, and the 2-year transplant-free survival rate has been reported to be approximately 40%. No medical treatment has been established for ventricular diastolic dysfunction; thus, heart transplantation is the only way to cure end-stage heart failure in patients with RCM. Herein, the pathology, etiology, clinical examinations, and medical management of pediatric patients with RCM were reviewed. This article includes a clinical case review of pediatric patients with RCM in our institute and the results of in vitro experiments using cardiac fibroblasts derived from patients with RCM and exome sequencing.

Congenital heart disease (CHD) is considered to be caused by multifactorial inheritance. Genetic variants and environmental factors have a complex effect on its onset. Chromosomal defects and single-gene disorders can also cause CHD. By analyzing syndromic CHDs and Mendelian inherited familial CHDs, many CHD-related genes have been identified. On the other hand, a large number of CHD-related genes are estimated to be undiscovered. The genetic basis of sporadic CHD also has not been elucidated. Over the last decade, owing to the advances in comprehensive DNA analysis techniques, our understanding of the genetic architecture of CHD is expanding. These advances include next-generation sequencers for nucleotide sequences and chromosomal microarray analysis for copy number abnormalities. Novel CHD-related genes have been reported from comprehensive genetic analyses of rare families and syndromes. Comprehensive genetic analysis of large cohorts have provided new areas of research, such as the identification of genes involved in the development of sporadic CHD and the analysis of epigenetic regulation in CHD development. This article reviews the history and prospects of comprehensive genetic analysis of CHD.

Objective: To investigate whether 12-lead electrocardiography at 1 month after birth can predict the prognosis of patients with perimembranous ventricular septal defect (VSD).

Methods: We divided 44 perimembranous VSD cases were divided into three groups according to the outcome at the age of 1 year as follows: naturally closed (n＝14), unclosed (n＝17), operative (n＝13) and compared their electrocardiographic findings with those at the age of 1 month. We also compared the operative group with the nonoperative groups (naturally closed and unclosed) and the naturally closed group with the non-naturally closed groups (operative and unclosed) to identify factors predictive of operation and natural closure, respectively.

Results: The results are shown by (naturally closed vs. unclosed vs. operative; p value). By comparing all the groups, we found that V1 positive T-wave (0％ vs. 11.8％ vs. 84.6％; p＜0.001) was significantly more frequent and the the frequency of the V5 R-wave (1.81 mV [0.72–2.88 mV] vs. 2.07 mV [0.90–3.77 mV] vs. 2.34 mV [1.55–3.85 mV]; p＝0.005) was significantly higher in the operative group. V1-positive T-wave (84.6％ vs. 6.5％; p＜0.001) was significantly more frequent and V1 R-wave (1.64 mV [0.60–4.73 mV] vs. 1.21 mV [0.29–2.62 mV]; p＝0.015) was significantly higher in the operative group than in the nonoperative group. In the naturally closed group, the V5 R-wave (1.81 mV [0.72–2.88 mV] vs. 2.13 mV [0.90–3.85 mV]; p＝0.025) and V5 S-wave (0.61 mV [0.35–1.55 mV] vs. 0.99 mV [0.26–2.98 mV]; p＝0.036) were significantly lower.

Conclusion: In patients with perimembranous VSD, 12-lead electrocardiography at 1 month of age may predict the prognosis at 1 year of age.

Background: With the improved outcomes of congenital heart diseases, the number of patients dying from heart failure in adulthood is increasing. Palliative care for chronic heart failure is becoming increasingly important, but only few studies have reported on pediatric palliative care for patients with heart failure.

Methods: Pediatric cardiac patients aged ＞10 years who died between 2000 and 2020 were included. The characteristics of the patients who died from heart failure, including their clinical symptoms, medications, ventilators, multi-professional conferences, informed consent, psychologist intervention, and chest compressions, were studied retrospectively.

Results: Seven patients died from heart failure, which was the most common cause of death. The median age at the time of death from heart failure was 15 years (range, 10–24 years). Multi-professional palliative conferences were held for two cases (29％), and informed consent was provided for one case. Sedation drugs were used in five patients (71％), but opioids were not used in any of the cases except for the orally intubated patients. Dyspnea was present in almost all the patients.

Conclusion: Palliative care for patients with pediatric heart failure is still developing, and a multi-professional palliative care team and system must be established.

The pathophysiology of coronary spastic angina (CSA), which rarely develops in childhood, remains unknown. A 10-year-old boy with a previous history of non-sustained chest pain at rest was admitted to the hospital because of a strong chest pain continuing for 30–40 minutes with a feeling of chest tightness during sleeping. Electrocardiography revealed an ST elevation in a wide range of leads, and myocardial enzyme levels were elevated. Echocardiography revealed no findings to suggest acute myocarditis or structural disease of the coronary artery. We made a diagnosis of CSA and started nitroglycerin therapy, with no pain recurrence. An acetylcholine provocation test induced diffuse spasm in all of the three coronary artery branches and ST elevation in V4–V6. We diagnosed the patient with CSA and started administration of a calcium channel blocker. No CSA recurrence was observed after the treatment. The reactive hyperemia index, which represents vascular endothelial function, was 1.17, as shown by reactive hyperemia-peripheral arterial tonometry. This value was far below the normal value (≥1.67). We suggest that systemic endothelial dysfunction might contribute to the development of CSA in childhood.

Interventional radiology (IVR) is a new effective therapeutic strategy for chylothorax. However, reports of IVR applied in children, especially neonates, are limited. Herein, we report the case of a 1-day-old neonate with complete transposition of the great arteries, ventricular septal defect, and right aortic arch. She underwent pulmonary artery banding at 13 days of age, and during thoracotomy, chylothorax was observed. We started tube feeding on postoperative day 3. On postoperative day 6, a considerable amount of chyle (170 mL/day) was drained; hence, a conservative treatment was introduced but was subsequently found to be ineffective. We performed intranodal lymphangiography (IL) on postoperative day 11. Consequently, the chylothorax improved 3 days later. Therefore, IL was effective for the management of neonatal postoperative chylothorax. In conclusion, IL is less invasive than surgical treatment and is effective against refractory chylothorax. However, further case reports of children and neonates who have undergone IL are necessary to confirm these findings.

A very low-birth-weight neonate was referred to our hospital because of total anomalous pulmonary venous connection (TAPVC) and extracardiac multiple anomalies, including high imperforate anus. The TAPVC was classified as type Ib, and a diagnosis of pulmonary venous obstruction was made. Furthermore, left unilateral renal agenesis and right multicystic dysplastic kidney were found. We discussed the treatment strategy with physicians from other departments. TAPVC was urgently corrected using a sutureless surgical technique on day 1 of life. During the management of the imperforate anus, the patient was deeply sedated to avoid aerophagia. Peritoneal dialysis was used to improve the edema postoperatively. Colostomy was performed on postoperative day 12. The patient was extubated on postoperative day 19. Later, the patient was diagnosed with cat eye syndrome by chromosome analysis. Cardiac catheterization at 8 months of age revealed pulmonary hypertension. Moreover, home oxygenation therapy and medications such as bosentan hydrate and tadalafil were administered. Consequently, the imperforate anus was radically corrected at 1 year of age. At 5 years of age, the patient is doing well with good renal function. This paper reports the operation and management of a very low-birth-weight neonate with critical congenital heart disease and extracardiac multiple anomalies.

Neonatal necrotizing enterocolitis (NEC) is a serious complication associated with congenital heart disease (CHD). Non-IgE-mediated gastrointestinal food allergy (non-IgE-GI-FA) in neonates and infants manifests as digestive symptoms, including emesis, diarrhea, melena, and abdominal distension, with occasional intestinal perforation and NEC. Previous studies showed the relationship between CHD and non-IgE-GI-FA, which suggests that a decrease in intestinal blood flow can be a risk factor of non-IgE-GI-FA in neonates and infants with CHD. We report the case of a neonate with transposition of the great arteries who developed NEC before an arterial switch operation (ASO) and non-IgE-GI-FA after the operation. The patient presented with bloody stools after the start of formula feeding and was diagnosed with NEC on the basis of abdominal radiography findings. After recovery from NEC, the patient underwent ASO. However, the patient presented with abdominal distension after formula feeding was restarted and was diagnosed with non-IgE-GI-FA on the basis of the findings of eosinophilia and positivity in an allergen-specific lymphocyte stimulation test. This demonstrates that postoperative digestive symptoms impose the possibility of non-IgE-GI-FA in neonates with CHD, especially in those with an NEC complication. Therefore, we infer that if gastrointestinal complications occur in patients with severe CHD, the possibility of non-IgE-GI-FA should be considered.